Variation name (cDNA level) | Variation name (protein level) | Variation status | Variation class |
c.1345G>A | p.Val449Ile | Heterozygous | Polymorphism |
wt codon | wt aa | mutant codon | mutant aa | mutational event | mutation type |
GTT | Val | ATT | Ile | G->A | Ts |
Structure | Key Residue (HCD) | Pyrimidin doublet | CpG |
EGF-like #04 | N-Term MMPs site | No | Yes |
At the mRNA level | On restriction map |
NA | New restriction site(s): none Lost restriction site(s): Mae II |
Conservation (0-1) | SIFT (0-1) | UMD-predictor (0-100) |
---|---|---|
0.83 | 1.00 (non pathogenous) | 35 (Polymorphism) |
Sample ID | Patient status | Gender | Transmission | Age of onset | Age of death | Geographic origin |
GER03HAN F0013 I03 | Proband | Male | NA | 15 years old | GERMANY |
Phenotypic group | Disease |
NA | Incomplete MFS |
Symptom | Severity |
C-Aortic insufficiency | severe |
C-Asc. aortic dilatation | |
no clinical data | |
O-No implication | |
S-Increased body length |
Reference ID | PubMed ID | Reference |
107 | 12402346 | Rommel K, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M. "Mutation screening of the fibrillin-1 (FBN1) gene in 76 unrelated patients with Marfan syndrome or Marfanoid features leads to the identification of 11 novel and three previously reported mutations". Hum Mutat. 2002 Nov;20(5):406-7. |